Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This case report describes 3 patients with Down syndrome and obstructive breathing problems, ages: 5 months, 15 months, and 22 years. The youngest one had normal cardiopulmonary function at birth, but soon developed a pulmonary hypertension. The next had a severe atrioventricular defect and additional pulmonary hypertension and there was little hope for her to survive heart surgery. The oldest one had had apneas since childhood with increasing severity, but was regarded as having normal heart function. All 3 were operated to relieve their breathing obstruction. The 5-month-old boy improved only slightly after an initial UPPP and had to be tracheotomized, which solved his problems. The tracheotomy could be removed when he was one year. The 15-month-old girl was cured of her breathing problems through an A + T and survived her heart surgery one month later. Tonsillectomy and UPPP was performed on the oldest patient. Following surgery, he had an episode of life-threatening bleeding and developed a DIC syndrome, and was critically ill for 18 days. After he recovered, his sleep apnea had improved. Once a myxoedema was diagnosed and treated, he made further progress. These cases stress the significance of early recognition of sleep apnea in children with Down syndrome and the importance of a careful preoperative investigation in collaboration with cardiologists.
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PMID:Down syndrome and sleep apnea--a therapeutic challenge. 183 Nov 85

Selective reduction in multiple gestation refers to abortion of specific fetuses, either because of congenital defect of grand multiple gestation. Fetal indications for which selective termination has been reported are Down syndrome, microcephaly, hemophilia A, spina bifida, thalassemia major and Tay-Sachs disease. Grand multiple gestations, defined as 4 or more fetuses in a pregnancy, have been selectively terminated in cases of 4-9 fetuses. Most couples choose to reduce multiple gestations to twin pregnancies. Very short women with multiple gestation are particularly at risk. Methods used have included needle aspiration of amniotic fluid, cardiac puncture and aspiration, and intrathoracic injection of KC1 or calcium gluconate. Potassium chloride is preferred because it is rapid, so results can be determined immediately without having to repeat the procedure. It is preferable to time the termination at 11 weeks' gestation to lower the risk of disseminated intravascular coagulation, which can result from absorption of fetal tissue. Most gynecologists prefer to select for fundal implantations. The ethical alternatives of this type of termination are either to abort the entire pregnancy, or risk the life of the mother as well as the life and well-being of all the fetuses. Most women with multiple gestations are those with history of infertility, who have gone to greater expense and emotional investment to become pregnant. Legally, selective reduction is a type of 1st trimester abortion, subject to institutional experimental protocols and patient's informed consent.
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PMID:Selective reduction in multiple gestation. 273 40

We present a case of a 64-year-old male, diagnosed to have acute promyelocytic leukemia with trisomy 21. He came to the hospital with bleeding secondary to disseminated intravascular coagulation. Promyelocytes in the blood and bone marrow contained abundant, prominent azurophilic granules. Cytogenetic studies revealed trisomy 21. The karyotypic abnormality reverted back to normal 46,XY, pattern after chemotherapy. The typical morphologic and cytogenetic features of acute promyelocytic leukemia are briefly discussed.
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PMID:Trisomy 21 in acute promyelocytic leukemia. 295 63

This report concerns an unusual case of thrombosis and hemorrhage in the choroid plexus caused by disseminated intravascular coagulation (DIC). The case is of special interest because it occurred in a patient with Down's syndrome. Nevertheless, there is no causal relation between Down's syndrome and the choroid plexus lesion except that mongols are susceptible to infections that trigger DIC. The association of choroid plexus thrombosis-hemorrhage and Down's syndrome is coincidental and does not indicate a basic vascular defect.
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PMID:Choroid plexus thrombosis and hemorrhage caused by disseminated intravascular coagulation in Down's syndrome. 623 Mar 3

Agranulocytosis caused by ethosuximide is extremely rare in children. Drug-induced agranulocytosis is an unexpected side effect of a drug, and delay in diagnosis of agranulocytosis can result in a fatal outcome. We experienced a case of a 16-month-old male infant with Down syndrome in whom fever appeared 16 days after the start of administration of ethosuximide and then severe pneumonia developed. Results of a blood test on admission showed a decreased leukocyte count of 1700/microl, and a hemogram showed that there were no granulocytes. The erythrocyte and thrombocyte counts were within normal ranges. The results of a bone marrow aspiration test showed that there was no production of any types of granulocytes. The patient required mechanical ventilation due to deterioration in his pneumonia and complication with disseminated intravascular coagulation, but the neutrophilic leukocytes began to increase from the 8th day after discontinuation of ethosuximide administration and start of treatment with granulocyte colony-stimulating factor, and the patient survived. The mechanism of onset in this case is thought to have been immunologic. Careful attention should be given to this type of agranulocytosis because of its sudden onset at 1-2 weeks after the start of administration of the causal drug. A drug-induced lymphocyte stimulation test was useful for diagnosis in this case, showing a positive reaction only for ethosuximide.
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PMID:Ethosuximide induced agranulocytosis. 1312 98

A transient myeloproliferative disorder (TMD) occurs in 10% of the infants with Down syndrome. While most cases resolve within a few months, in 20% of them TMDs are life-threatening or fatal. We encountered 4 patients with TMD, including 1 patient who died of liver failure and disseminated intravascular coagulation. Suspecting involvement of proinflammatory cytokines, we serially assayed them in patients' sera. Cytokines were significantly more abundant in patients than in controls. Interleukins 1 and 2, tumor necrosis factor alpha, interferon gamma, and granulocyte-macrophage colony-stimulating factor were greatly increased, especially in the infant who died. Sustained cytokinemia is likely to participate in TMD pathophysiology, and very high serum concentrations might predict a poor outcome.
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PMID:Proinflammatory cytokinemia associated with transient myeloproliferative disorder in down syndrome. 1467 34

We report a male infant with Down syndrome who had a transient myeloproliferative disorder associated with skin lesions. He was transferred to a neonatal intensive care unit because of low body weight, fetal edema, disseminated intravascular coagulation, and 10% blast cells in the peripheral blood. On postnatal day (PD) 1, erythema with small papules, vesicles, and pustules appeared on the entire body. A smear preparation from the pustules on PD 2 showed 10% blast cells. A biopsy specimen taken on PD 5 revealed subcorneal pustules containing neutrophils and eosinophils. Genetic analyses detected a somatic mutation (197G>T, Glu295Stop) in exon 2 of GATA-1. On PD 10, the eruptions resolved spontaneously and the population of blast cells in peripheral blood decreased to 1%. The number of blast cells in pustules decreased markedly after three days. Therefore, we recommend that cytologic examination should be performed as early as possible.
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PMID:Transient myeloproliferative disorder with vesiculopustular eruption: Early smear is useful for quick diagnosis. 1938 30